Normal aging modulates the neurotoxicity of mutant huntingtin.

Détails

ID Serval
serval:BIB_AB2FE30D4963
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Normal aging modulates the neurotoxicity of mutant huntingtin.
Périodique
Plos One
Auteur⸱e⸱s
Diguet E., Petit F., Escartin C., Cambon K., Bizat N., Dufour N., Hantraye P., Déglon N., Brouillet E.
ISSN
1932-6203 (Electronic)
ISSN-L
1932-6203
Statut éditorial
Publié
Date de publication
2009
Volume
4
Numéro
2
Pages
e4637
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov'tPublication Status: ppublish
Résumé
Aging likely plays a role in neurodegenerative disorders. In Huntington's disease (HD), a disorder caused by an abnormal expansion of a polyglutamine tract in the protein huntingtin (Htt), the role of aging is unclear. For a given tract length, the probability of disease onset increases with age. There are mainly two hypotheses that could explain adult onset in HD: Either mutant Htt progressively produces cumulative defects over time or "normal" aging renders neurons more vulnerable to mutant Htt toxicity. In the present study, we directly explored whether aging affected the toxicity of mutant Htt in vivo. We studied the impact of aging on the effects produced by overexpression of an N-terminal fragment of mutant Htt, of wild-type Htt or of a beta-Galactosidase (beta-Gal) reporter gene in the rat striatum. Stereotaxic injections of lentiviral vectors were performed simultaneously in young (3 week) and old (15 month) rats. Histological evaluation at different time points after infection demonstrated that the expression of mutant Htt led to pathological changes that were more severe in old rats, including an increase in the number of small Htt-containing aggregates in the neuropil, a greater loss of DARPP-32 immunoreactivity and striatal neurons as assessed by unbiased stereological counts.The present results support the hypothesis that "normal" aging is involved in HD pathogenesis, and suggest that age-related cellular defects might constitute potential therapeutic targets for HD.
Mots-clé
Aging/physiology, Animals, Corpus Striatum/enzymology, Corpus Striatum/pathology, Dopamine and cAMP-Regulated Phosphoprotein 32/genetics, Nerve Tissue Proteins/genetics, Nerve Tissue Proteins/physiology, Nuclear Proteins/genetics, Nuclear Proteins/physiology, Promoter Regions, Genetic, Rats, beta-Galactosidase/genetics
Pubmed
Web of science
Open Access
Oui
Création de la notice
13/12/2011 17:15
Dernière modification de la notice
20/08/2019 16:15
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